Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Although seen rarely in Switzerland, schistosomiasis is a parasitosis affecting 200 to 250 million people round the world, mainly in tropical and subtropical regions of Africa, Asia, Central and South America. Depending on the parasitic species, the ureters and the bladder (S. haematobium) or the intestine and the liver (S. mansoni, S. japonicum, S. mekongi) are primarily involved. Other organs may be affected (lung, kidneys and central nervous system). Hepatosplenic schistosomiasis represents a special form of chronic infection by S. mansoni, S. japonicum or S. mekongi predominantly occurring in adolescents heavily and repeatedly infected during childhood, together with an additional genetic predisposition for the disease. Hepatosplenic schistosomiasis on a worldwide scale is one of the most prevalent causes of portal hypertension in man. We describe a 33-year-old Portuguese female with mansonian hepatosplenic schistosomiasis 12 years after leaving Africa, who had hepatosplenomegaly, portal hypertension, esophageal varices and hypersplenism. Splenomegaly and slight anemia had been known for years without prompting further work-up. Two months before diagnosis she had been delivered of a normal child after pregnancy without portal-hypertensive complications, namely esophageal hemorrhage. Because of placenta accreta, however, erythrocyte transfusion had been performed after delivery and was possibly responsible for hepatitis C found later on. Pathophysiology, clinical findings and therapy of the disease are discussed.
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PMID:[Hepatosplenic schistosomiasis: case report and clinical review]. 825 82

The literature on the assessment of morbidity due to Schistosoma mansoni infection is updated. Imaging techniques such as ultrasonography, echodoppler cardiography, computerized tomography (CT scan) and magnetic resonance imaging (MRI) introduced a new perspective, and expanded our knowledge on morbidity. Three well-defined syndromes caused by schistosomiasis mansoni have been described: the stage of invasion, acute schistosomiasis (Katayama fever), and chronic schistosomiasis. Complications of the acute and chronic syndromes have also been reported: pulmonary hypertension, neuroschistosomiasis, association with Salmonella, association with Staphylococci, viral hepatitis B, glomerulonephritis. In most individuals with hepatosplenic schistosomiasis the spleen is increased in size. Hepatosplenic schistosomiasis can, however, occur without splenomegaly. The definition of hepatosplenic schistosomiasis in endemic areas as the finding of S. mansoni eggs in the stools in an individual with hepatosplenomegaly is not satisfactory anymore. Many aspects of morbidity are expected to change after schistosomiasis control. Some are expected to change quickly (worm burden, Salmonella bacteremia, hepatosplenic schistosomiasis in children) whereas others shall remain for years (pulmonary hypertension, glomerulonephritis, neuroschistosomiasis). Intestinal schistosomiasis in individuals with low worm burdens is very difficult to diagnose and therefore laborious to control.
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PMID:Schistosoma mansoni: assessment of morbidity before and after control. 1099 26

Schistosomiasis mansoni is a widespread parasitic disease in the Brazilian territory that affects over 8 million individuals. Hepatosplenic schistosomiasis is a serious clinical presentation of this disease, associated with splenomegaly, liver fibrosis, and portal hypertension, and is responsible for approximately 7% of schistosomotic patients. The surgical treatment of portal hypertension in schistosomotic patients has distinct features when compared with cirrhotic patients, mostly because hepatic function is preserved in schistosomotic liver disease. Therefore, when attempting to reduce the portal pressure, the surgeon must be aware that the surgery might interfere with hepatic perfusion, and consequently with hepatic function. The aim of this study was to report the results achieved with splenectomy, division of the left gastric vein, devascularization of great gastric curvature, and postoperative endoscopic variceal sclerosis, as a surgical option to esophageal varices in hepatosplenic schistosomiasis. A total of 111 patients were studied, and the following is a list of inclusion criteria: age >16 years, history of gastrointestinal (GI) bleeding, presence of esophageal varices on preoperative endoscopy, hematocrit >22% and prothrombin enzymatic activity >50%, negative viral hepatitis on serologic tests (anti-HBV and anti-HCV), and definition, after liver biopsy, of exclusive schistosomotic liver disease. The following list includes exclusion criteria used: presence of liver disease other than schistosomotic, history of alcohol abuse, and preoperative thrombosis of the portal vein. The rebleeding rate was 14.4% during a mean 30-month follow-up period; portal vein thrombosis was 13.2%, and there was a global mortality of 5.4%. Gastric varices were present in 46.9% of the patients; for those patients, a gastrotomy and running suture of the varices achieved an eradication rate of the varices of 75.6%. The degree of periportal fibrosis was also analyzed. Periportal fibrosis staging revealed that patients with class II or III liver fibrosis had a significant increased risk of recurrent GI bleeding when compared with patients with class I liver fibrosis. Despite the elevation on alanine aminotransferase (ALT) and aspartate aminotransferase (AST), most other liver function tests showed no alteration or were corrected after surgery. We conclude that splenectomy, division of the left gastric vein, devascularization of great gastric curvature, and postoperative endoscopic variceal sclerosis showed good results globally and should be considered as therapeutic options in the treatment of hepatosplenic schistosomiasis.
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PMID:Surgical treatment of schistosomal portal hypertension. 1189 Mar 33

We report a case of hepatosplenic schistosomiasis with portal hypertension and variceal bleeding in an immigrant patient from Egypt, coinfected with Strongyloides stercoralis. The diagnosis was based on the following: (a) identification of Schistosoma mansoni ova in the stools and colonic biopsy specimens, (b) portal hypertension and esophageal varices with normal liver function and the absence of hepatic cirrhosis stigmata, (c) history of migration from an endemic area and (d) ultrasonographic findings of spleen and liver enlargement, fibrosed portal tracts, and normal lobular architecture of liver parenchyma. Hepatosplenic schistosomiasis should be suspected in any patient from an endemic area who has splenomegaly, portal hypertension, and esophageal varices bleeding in the absence of stigmata of liver cirrhosis and hepatic insufficiency. Coinfection with S. stercoralis could be attributed to common epidemiological features of the parasites and the patient's habits.
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PMID:Coinfection of Schistosoma mansoni and Strongyloides stercoralis in a patient with variceal bleeding. 1609 3

Hepatosplenic schistosomiasis was the first human disease in which the possibility of extensive long standing hepatic fibrosis being degraded and removed has been demonstrated. When such changes occurred, the main signs of portal hypertension (splenomegaly, esophageal varices) progressively disappeared, implying that a profound vascular remodeling was concomitantly occurring. Hepatic vascular alterations associated with advanced schistosomiasis have already been investigated. Obstruction of the intrahepatic portal vein branches, plus marked angiogenesis and compensatory hyperplasia and hypertrophy of the arterial tree are the main changes present. However, there are no data revealing how these vascular changes behave during the process of fibrosis regression. Here the mouse model of pipestem fibrosis was used in an investigation about these vascular alterations during the course of the infection, and also after treatment and cure of the disease. Animals representing the two polar hepatic forms of the infection were included: (1) "isolated granulomas" characterized by isolated periovular granulomas sparsely distributed throughout the hepatica parenchyma; and (2) 'pipestem fibrosis' with periovular granulomas and fibrosis being concentrated within portal spaces, before and after treatment, were studied by means of histological and vascular injection-corrosion techniques. Instances of widespread portal vein obstruction of several types were commonly found in the livers of the untreated animals. These obstructive lesions were soon repaired, and completely disappeared four months following specific treatment of schistosomiasis. Treatment was accomplished by the simultaneous administration of praziquantel and oxamniquine. The most impressive results were revealed by the technique of injection of colored masses into the portal system, followed by corrosion in strong acid. The vascular lesions of non-treated pipestem fibrosis were represented in the plastic casts by considerable diminution of the fine peripheral portal vein radicles, plus dilatation of periportal collaterals. Four months after treatment, this last picture appeared replaced by tufts of newly interwoven vessels formed along the main portal vein branches, disclosing a strong angiomatoid reparative change. Understanding about the cellular elements at play during fibro-vascular repairing changes of hepatic schistosomiasis represents a matter of considerable scientific and conceptual importance. At present time one may only speculate about the participation of some type of natural stem-cell capable of restoring the diseased liver back to normal once the cause of the disorder has been eliminated.
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PMID:Remodeling of hepatic vascular changes after specific chemotherapy of schistosomal periportal fibrosis. 1730 80